1/16. Laminar cortical necrosis in central nervous system lupus: sequential changes in MR images.A 44-year-old woman with systemic lupus erythematosus developed central nervous system lupus presenting with headache, fever, cloudiness of consciousness, and psychotic symptoms. T1-weighted and proton MR images showed laminar high intensity lesions in the parietal and temporal cortex bilaterally. T2-weighted images of the lesions showed low signal intensity. Treatment with corticosteroids alleviated the clinical symptoms within 7 months. The low T2 and high T1 signal abnormalities disappeared in a year and in 5 years, respectively. The laminar cortical lesions on MR images were suggested to represent cortical necrosis associated with central nervous system lupus.- - - - - - - - - - ranking = 1keywords = ear (Clic here for more details about this article) |
2/16. Neuro-Behcet's disease.Behcet's disease (BD) is a multifocal disorder with an immunogenetic basis, which persists over many years. Initial descriptions mentioned oral and genital ulcers with uveitis. Later a number of other manifestations were added, like skin, joint and neurological. The involvement of nervous system (Neuro-Behcet's) is reportedly uncommon. We hereby report four cases of Neuro-Behcet's, i.e.; two cases of strokes involving multiple areas of the central nervous system and two cases had features of benign intracranial hypertension. All cases had mucocutaneous lesions or other system involvement. Cases satisfied the international study group criteria for diagnosis of BD. All cases were pathergy test positive. In comparison with the literature from turkey and greece, which reports a high pathergy positivity, reports from india have shown only few cases to be positive. The prognosis of Neuro-Behcet used to be poor but has recently been improved with reduced mortality, although whether this can be attributed to treatment with steroids and/or cytotoxic agents remains uncertain.- - - - - - - - - - ranking = 0.25keywords = ear (Clic here for more details about this article) |
3/16. Autoimmune sensorineural hearing loss improved by tumor necrosis factor-alpha blockade: a case report.Autoimmune inner ear disease is a treatable cause of sensorineural hearing loss and it is important for physicians and hearing health professionals to recognize that early diagnosis and proper management strategies may result in stabilization and improvement in hearing. The pathogenesis of autoimmune sensorineural hearing loss remains unclear but antibodies directed against the inner ear and/or cellular effectors have been proposed. Therefore, immunosuppressive drugs such as steroids and methotrexate are administered to interfere with the progression of hearing loss and in some cases have been found to improve hearing. We report herein the history of a patient who was treated by systemic administration of anti-tumor necrosis factor-alpha antibodies for Crohn's disease and who also had associated sensorineural hearing loss. Audiometric follow-up revealed not only the efficacy of tumor necrosis factor-alpha blockade in arresting the hearing loss but also an improvement in hearing of 15 dB on average across all frequencies. Hearing remained stable afterwards.- - - - - - - - - - ranking = 28560.817597204keywords = inner ear disease, ear disease, inner ear, inner, ear (Clic here for more details about this article) |
4/16. Molecular tracking of antigen-specific T cell clones in neurological immune-mediated disorders.T cells recognizing self or microbial antigens may trigger or reactivate immune-mediated diseases. Monitoring the frequency of specific T cell clonotypes to assess a possible link with the course of disease has been a difficult task with currently available technology. Our goal was to track individual candidate pathogenic T cell clones, selected on the basis of previous extensive studies from patients with immune-mediated disorders of the CNS, including multiple sclerosis, HTLV-I associated myelopathy/tropical spastic paraparesis (HAM/TSP) and chronic lyme neuroborreliosis. We developed and applied a highly specific and sensitive technique to track single CD4( ) and CD8( ) T cell clones through the detection and quantification of T cell receptor (TCR) alpha or beta chain complementarity-determining region 3 transcripts by real-time reverse transcriptase (RT)-PCR. We examined the frequency of the candidate pathogenic T cell clones in the peripheral blood and CSF during the course of neurological disease. Using this approach, we detected variations of clonal frequencies that appeared to be related to clinical course, significant enrichment in the CSF, or both. By integrating clonotype tracking with direct visualization of antigen-specific staining, we showed that a single T cell clone contributed substantially to the overall recognition of the viral peptide/MHC complex in a patient with HAM/TSP. T cell clonotype tracking is a powerful new technology enabling further elucidation of the dynamics of expansion of autoreactive or pathogen-specific T cells that mediate pathological or protective immune responses in neurological disorders.- - - - - - - - - - ranking = 0.25keywords = ear (Clic here for more details about this article) |
5/16. Thyrotoxic autoimmune encephalopathy: a repeat positron emission tomography study.Thyroid related autoantibodies have been related to the development of encephalopathy, known as Hashimoto's encephalopathy. However, their relation with the encephalopathy occurring in patients with Graves' disease has not been well established. The case is reported of a 51 year old woman presenting with subacute progressive dementia with evidence of hyperthyroidism. She had Graves' disease associated with high titres of thyroid related autoantibodies. Her encephalopathy was not improved by antithyroid drugs, but promptly responded to corticosteroid treatment, and stabilised with a gradual reduction of thyroid related autoantibody titres. brain positron emission tomography initially showed a diffuse and multifocal cerebral hypometabolism with subsequent normalisation on her clinical recovery, which was consistent with the acute and reversible cerebral inflammation probably mediated by autoimmune mechanisms.- - - - - - - - - - ranking = 0.25keywords = ear (Clic here for more details about this article) |
6/16. The spectrum of autoimmune autonomic neuropathies.We analyzed the clinical characteristics of 18 patients (13 female, 5 male) who had autoimmune autonomic neuropathy (AAN) and ganglionic acetylcholine receptor (AChR) autoantibodies. Mean age was 61.4 years (standard deviation, 12.0 years). Ten patients had subacute symptom onset, six with an antecedent event. Eight patients had chronic AAN, characterized by insidious symptom onset, without antecedent event, and gradual progression. A majority of patients with high antibody values (>1.00 nmol/L) had a combination of sicca complex (marked dry eyes and dry mouth), abnormal pupillary light response, upper gastrointestinal symptoms, and neurogenic bladder. Chronic AAN segregated into two subgroups. One subgroup (N = 4) had low antibody titer (0.09 /- 0.01 nmol/L) and a paucity of cholinergic symptoms. It was indistinguishable from pure autonomic failure. The other subgroup (N = 4) had high antibody titer (11.6 /- 2.08 nmol/L), sicca complex, abnormal pupils, and neurogenic bladder; three had severe upper gastrointestinal dysfunction. Higher antibody titers correlated with greater autonomic dysfunction and more frequent cholinergic dysautonomia. These observations expand the clinical spectrum of AAN to include chronic cases, some being indistinguishable from pure autonomic failure, and support the concept that ganglionic AChR antibodies are important diagnostically and pathophysiologically in acquired dysautonomia.- - - - - - - - - - ranking = 0.5keywords = ear (Clic here for more details about this article) |
7/16. Neuropathology and binding studies in anti-amphiphysin-associated stiff-person syndrome.The authors report a 71-year-old woman with amphiphysin-associated paraneoplastic stiff-person syndrome, opsoclonus, and encephalopathy. The patient's symptoms temporarily responded to plasmapheresis in parallel with a decline of serum anti-amphiphysin antibody titers. Later, the encephalopathy progressed rapidly and the patient died. Binding studies and the detection of autoantibodies in the patient's CNS as well as the treatment response suggest a pathogenic role of the anti-amphiphysin antibodies.- - - - - - - - - - ranking = 0.25keywords = ear (Clic here for more details about this article) |
8/16. Autoimmune optic neuropathy with anticardiolipin antibody mimicking multiple sclerosis in a child.PURPOSE: To demonstrate that autoimmune optic neuropathy (AON) may occur in the pediatric population. DESIGN: Interventional case report. methods: A 4-year-old developed four episodes of bilateral optic neuritis with mild concurrent weakness, ataxia, or dizziness; one episode of unilateral optic neuritis; and one episode of weakness over a period of 4 years. RESULTS: Autoimmune optic neuropathy was diagnosed because of the presence of anticardiolipin antibody and an abnormal skin biopsy with thrombin and immunoreactant deposition. She was treated with corticosteroids, aspirin, and gammaglobulin. This diminished the frequency and intensity of her attacks. CONCLUSION: This case represents the diagnosis and treatment of AON in a child.- - - - - - - - - - ranking = 0.5keywords = ear (Clic here for more details about this article) |
9/16. noise-induced autoimmune sensorineural hearing loss.Typically, autoimmune sensorineural hearing loss has been described as a slowly progressive, asymmetric hearing loss that is responsive to medications traditionally used in the treatment of other autoimmune conditions. These medications include steroids and cytotoxic drugs. Inciting factors in autoimmune inner ear disease are rarely cited. We describe a case of episodic sudden hearing loss triggered consistently by environmental noise. The hearing loss was responsive to steroids at the time of each occurrence and was determined to be autoimmune. This case raises questions about the relationship between autoimmune inner ear disease and sensitivity to environmental noise that warrant further research.- - - - - - - - - - ranking = 56790.095562884keywords = inner ear disease, ear disease, inner ear, inner, ear (Clic here for more details about this article) |
10/16. Chronic neuropathy with IgM anti-ganglioside antibodies: lack of long term response to rituximab.Two patients with chronic motor neuropathy, high antiganglioside antibody (AGA) titers, and a declining response to IV immunoglobulins were treated with rituximab at a standard dose. The drug was well tolerated and effectively eliminated peripheral B cells (CD20 ), but AGA titers continued significantly high. No clinical improvement was detected during the 1-year follow-up.- - - - - - - - - - ranking = 0.25keywords = ear (Clic here for more details about this article) |
| | Next -> |